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Ependymal Lined Paraventricular Cerebral Cysts; a Report of Three Cases

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Ependymal Lined Paraventricular Cerebral Cysts; a Report of Three Cases J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.36.4.611 on 1 August 1973. Downloaded from Journial of Neurology, Neurosurgery, and Psychiatry, 1973, 36, 611-617 Ependymal lined paraventricular cerebral cysts; a report of three cases D. C. BOUCH, I. MITCHELL, AND A. F. J. MALONEY From the Neuropathology Laboratory, Department ofPathology, University of Edinburgh and Milesmark Hospital, Dunfermline SUMMARY Three cases are reported, each with a benign ependymal-lined cyst which produced clinical signs and symptoms simulating cerebrovascular disease or cerebral neoplasm. The pathologi- cal features are described and their histogenesis discussed. Intracranial cysts lined by ependyma which are embolus with the main pathological features con- large enough to give rise to symptoms are rare. fined to the brain. Virtually all references to ependymal lined cysts BRAIN Macro The brain showed swelling of the in the literature relate to the colloid or para- Protected by copyright. physeal cyst of the third ventricle. right frontal lobe, flattening of the overlying gyri, and shift of the midline structures to the left. Occupy- This communication describes three cases in ing most of the central white matter of the right each of which a large cyst with watery content frontal lobe was a unilocular cyst (Fig. 1) compres- was found in the centrum semi-ovale. In no case sing, but not communicating with, the lateral ventricle could communication with the ventricular system and displacing the anterior corpus striatum down- be demonstrated. All presented with severe neuro- wards. The cyst, which measured 60 x 4-0 cm in logical signs and symptoms. None was diagnosed during life. Two were thought to have a neo- plasm and the third was considered to suffer from cerebrovascular disease. As the pathological de- scription will demonstrate, the cysts were non- neoplastic, probably developmental in origin, and should have been amenable to surgery. http://jnnp.bmj.com/ CASE 1 (NP.871) (RIE.33/38) (Dr. A. C. P. Campbell and Dr. I. W. G. Hill) This 64 year old woman presented in January 1938, having experienced headaches and a feeling of pressure on the top of her head for one year. Over the last two months there was a gradual on September 23, 2021 by guest. deterioration of memory and mental faculties with the onset of drowsiness and confusion. Terminally, her condition deteriorated rapidly necessitating her admission to hospital where she became comatose, dying two weeks later. Details of clinical investiga- tions are lacking but she was considered to have sus- FIG. 1. Case 1. Coronal slice of cerebrum at level of tained cerebral optic chiasma showing a large, smooth-surfaced, uni- thrombosis. locular cyst in the white matter of the right frontal Post mortem examination showed that the im- lobe. Note compression and displacement ofthe ventri- mediate cause of death was a massive pulmonary cular system and rotation of the right basal ganglia. 611 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.36.4.611 on 1 August 1973. Downloaded from 612 D. C. Bouch, L Mitchell, and A. F. J. Maloney maximum diameter in the coronal plane and con- tained clear watery fluid, was separated from the right lateral ventricle by white matter 01 cm thick and from the subarachnoid space over the frontal lobe convexity by white matter and cortex 0-8 cm thick. Its wall was smooth and thin. 't The arteries were healthy. SF~~~ Micro The cyst wall was composed of white matter ....a lined on most of its inner aspect by a single layer of +.p low columnar or cubical epithelial cells, some of which were ciliated (Fig. 2) and contained blepharo- * the .. plasts. In one part of the wall, beneath epithelial 4 * *. lining, there was a small collection of tubules and ..... canaliculi lined by similar, but flattened cells (Fig. 3). .. p X The white matter showed minimal gliosis particularly 9. from those areas denuded of epithelium. There was no evidence of haemorrhage, haemosiderin, or neoplasm. COMMENT It was considered that this cyst was ....0 WI simple, lined by ependyma, and probably of developmental origin. Protected by copyright. .,;7. x S~~~~S CASE 2 4 4 (NPA.115/71) This 60 year old woman presented .- a in April having noticed weakness of her left arm and dragging of her left leg for one year. This later be- FIG. 2. Case 1. Part of cyst wall linied by ciliated came associated with minor left-sided sensory ependyma. H and E, x 325. changes, slurring of speech, increased urinary fre- t http://jnnp.bmj.com/ -Ir. el .j'3. V!'. AWL ':. -::..:,......w FIG. 3. Case 1. Part of cyst wall showing subjacent tubules lined ... # by H and E, V.- -^ ;. flattened ependyma. AL. "O.: ..,. AP" 9-.. x 275. on September 23, 2021 by guest. NVik. *.. VI5. .IEWrl W.. '... .::. ... V*4ww-OW . J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.36.4.611 on 1 August 1973. Downloaded from Ependymal lined paraventricular cerebral cysts; a report of three cases 613 minimal left-sided weakness and also revealed in- creased tendon reflexes in the left upper limb, a slightly spastic gait, dysarthria, mental slowness, lethargy, and poor concentration. There was no abnormality of the cardiovascular system and the fundi were normal. It was originally thought that she had an intracerebral space-occupying lesion but plain skull radiographs, electroencephalogram, lum- bar cerebrospinal fluid, and a cerebral isotope scan were normal. Other system investigations revealed equivocal thyroid function tests; the 48 hour uptake of 131iodine was consistent with, but not diagnostic of, hypothyroidism; the serum protein-bound iodine was normal. By now the only remaining clinical abnormalities were mental slowness andJlethargy and treatment with thyroxin was started. There was marked improvement in her condition. She be- - -1 was home after .". ir, came alert and active and allowed N . '..j two weeks' further observation. X .. Three days after discharge from hospital, left- sided weakness and headaches recurred. Her con- %,.0. -4, dition rapidly deteriorated and she was readmitted r.,- to hospital 12 hours later in coma. The left limbs were flaccid, the right spastic, and both plantar Protected by copyright. responses were extensor. Both pupils were fixed, FIG. 4. Case 2. Depressed area of cortex (S) in the the right dilated and the left constricted. There was posterior par-t of the right frotital convexity which no papilloedema. Her condition continued to overlay the partially collapsed intracerebral cyst seeni deteriorate and she died 12 hours after readmission. in Fig. 5. The bridging arachnoid mater has been re- Post mortem examination revealed pathological mnoved. x 05. changes in the brain only. quency, and occasional incontinence. Over the last BRAIN Macro The leptomeninges appeared three months intermittent frontal headaches had healthy except for slight brownish discolouration in become a prominent feature along with some diffi- relation to a sunken area of cortex bridged over by culty in recalling recent events. There was no history arachnoid mater and measuring 6-0 x 5 5 cm across of head injury. and 2 0 cm deep located in the right frontoparietal After hospital admission examination confirmed region close to the falx (Fig. 4). It appeared to have http://jnnp.bmj.com/ FIG. 5. Case 2. Coronal slice of cere- brum showinig the partially collapsed on September 23, 2021 by guest. O intracerebral cyst (C) and overlying lepressed cortex (S). x 0 7. J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.36.4.611 on 1 August 1973. Downloaded from 614 D. C. Bouchl, L Mitchlell, and A. F. J. Maloney W,0 . j,W.So > ; } ^ ,tS*::APrfit FIG. 6. Case 2. Part of cyst wall with layer of collagen *S re §lAw' s' iz *:_f t ' 2 interposed between flattened *_ * *s vS; SS§|iFet - 8tts~~~~~4 ependyma and slightly gliotic white matter. H and E, x 300. .7- W ' +'4 resulted from collapse of an underlying intracerebral in January 1972 having been essentially well until cyst, a tiny opening being seen in the floor through seven months before when she began to feel lost andProtected by copyright. which clear fluid issued. Section of the brain showed confused. After an initial slight improvement her a large, partially collapsed unilocular cyst situated condition deteriorated with the development of in the white matter of the supramedial quadrant of headaches and a right hemiparesis. Later, increasing the posterior aspect of the right frontal lobe (Fig. 5). drowsiness and a Parkinsonian type of tremor of This cyst communicated with the subarachnoid space the right limbs became apparent. via the small opening already noted in the cortex On admission to hospital, examination showed but nowhere did it communicate with the ventricular that her hemiparesis was accompanied by minor system. The cyst measured 6 0 x 4-0 cm in the coronal sensory deficits and there was a right homonymous plane at the level of the mamillary bodies and had a hemianopia. Lumbar cerebrospinal fluid and plain thin wall with a smooth glistening lining. The ventri- radiographs of the skull and chest were normal. cular system was compressed and displaced from An electroencephalogram indicated a left cerebral right to left with the right basal ganglia rotated space-occupying mass which appeared to be lying downwards and laterally. The cyst had extended into in the posterior frontal region. the convolutional white matter and, bounded by Craniotomy and careful needling of the left cortex, had crossed the midline under the falx. posterior frontal region failed to identify any lesion. The arteries were healthy. She failed to recover consciousness after operation http://jnnp.bmj.com/ and gradually became comatose, remaining so until Micro The wall of the cyst was lined internally her death two weeks later.
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